van Beek Dirk-Jan, Verschuur Anna Vera D, Brosens Lodewijk A A, Valk Gerlof D, Pieterman Carolina R C, Vriens Menno R
Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Internal Mail Number G.04.228, PO Box 85500, Utrecht 3508 GA, the Netherlands.
Department of Pathology, University Medical Center Utrecht, Internal Mail Number G02.5.26, PO Box 85500, Utrecht 3508 GA, the Netherlands. Electronic address: https://twitter.com/annaveraverschu.
Surg Oncol Clin N Am. 2023 Apr;32(2):343-371. doi: 10.1016/j.soc.2022.10.010.
Pancreatic neuroendocrine tumors (PNETs) occur in < 1/100,000 patients and most are nonfunctioning (NF). Approximately 5% occur as part of multiple endocrine neoplasia type 1. Anatomic and molecular imaging have a pivotal role in the diagnosis, staging and active surveillance. Surgery is generally recommended for nonfunctional pancreatic neuroendocrine tumors (NF-PNETs) >2 cm to prevent metastases. For tumors ≤2 cm, active surveillance is a viable alternative. Tumor size and grade are important factors to guide management. Assessment of death domain-associated protein 6/alpha-thalassemia/mental retardation X-linked and alternative lengthening of telomeres are promising novel prognostic markers. This review summarizes the status of surveillance and nonsurgical management for small NF-PNETs, including factors that can guide management.
胰腺神经内分泌肿瘤（PNETs）在每10万名患者中的发病率低于1例，且大多数为无功能性（NF）。约5%的病例是多发性内分泌肿瘤1型的一部分。解剖学和分子影像学在诊断、分期及主动监测中起着关键作用。对于直径大于2cm的无功能性胰腺神经内分泌肿瘤（NF-PNETs），一般建议手术以预防转移。对于直径≤2cm的肿瘤，主动监测是一种可行的选择。肿瘤大小和分级是指导治疗的重要因素。死亡结构域相关蛋白6/α地中海贫血/智力发育迟缓伴X连锁和端粒替代延长的评估是很有前景的新型预后标志物。本文综述了小的NF-PNETs的监测及非手术治疗现状，包括可指导治疗的因素。
